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Extramedullary plasmacytoma

MedGen UID:
75843
•Concept ID:
C0278619
•
Neoplastic Process
Synonyms: EP; Extramedullary Plasmacytoma; extramedullary plasmacytoma; Extramedullary plasmacytoma (clinical); extramedullary plasmacytoma (clinical); Extraosseous Plasmacytoma; extraosseous plasmacytoma; Malignant plasma cell neoplasm, extramedullary plasmacytoma; plasmacytoma, extramedullary; Plasmacytoma, extramedullary (not occurring in bone); plasmacytoma, extramedullary (not occurring in bone)
SNOMED CT: Extramedullary plasmacytoma (128921005); Plasmacytoma, extramedullary (not occurring in bone) (128921005); Extramedullary plasmacytoma (188718006); Extramedullary plasmacytoma (clinical) (188718006); Malignant plasma cell neoplasm, extramedullary plasmacytoma (188718006)
 
Monarch Initiative: MONDO:0002754

Definition

A localized malignant neoplasm arising in an extraosseous site. It is composed of clonal (malignant) plasma cells forming a tumor mass. It most frequently involves the oropharynx, nasopharynx, sinuses, and larynx. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVExtramedullary plasmacytoma

Professional guidelines

PubMed

Solitary Plasmacytoma: a Review of Diagnosis and Management.
Pham A, Mahindra A
Curr Hematol Malig Rep 2019 Apr;14(2):63-69. doi: 10.1007/s11899-019-00499-8. PMID: 30788667
Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel.
Caers J, Paiva B, Zamagni E, Leleu X, Bladé J, Kristinsson SY, Touzeau C, Abildgaard N, Terpos E, Heusschen R, Ocio E, Delforge M, Sezer O, Beksac M, Ludwig H, Merlini G, Moreau P, Zweegman S, Engelhardt M, Rosiñol L
J Hematol Oncol 2018 Jan 16;11(1):10. doi: 10.1186/s13045-017-0549-1. PMID: 29338789Free PMC Article
Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group.
International Myeloma Working Group
Br J Haematol 2003 Jun;121(5):749-57. PMID: 12780789

Recent clinical studies

Etiology

Extramedullary plasmacytoma: Tumor occurrence and therapeutic concepts-A follow-up.
Holler A, Cicha I, Eckstein M, Haderlein M, Pöttler M, Rappl A, Iro H, Alexiou C
Cancer Med 2022 Dec;11(24):4743-4755. Epub 2022 May 16 doi: 10.1002/cam4.4816. PMID: 35578404Free PMC Article
Extramedullary Plasmacytoma: Long-Term Clinical Outcomes in a Single-Center in China and Literature Review.
Zhu X, Wang L, Zhu Y, Diao W, Li W, Gao Z, Chen X
Ear Nose Throat J 2021 May;100(4):227-232. Epub 2020 Sep 17 doi: 10.1177/0145561320950587. PMID: 32941076
Mexican Concensus of Multiple Myeloma.
de la Peña-Celaya JA, Aguilar-Luevano J, Alcivar-Cedeño LM, Álvarez-Vera JL, Anaya-Cuellar I, Añorve-Hernández E, Arana-Luna LL, Arteaga-Ortíz L, Báez-Islas PE, Banda-García LI, Bates-Martín RA, Campa-Monroy DI, Cardiel-Silva M, Castillo-Salas ÁJ, Cota-Rangel X, Díaz-Vargas G, Espitia-Ríos ME, Estrada-Domínguez P, Fermín-Caminero D, García-Camacho A, García-Castillo C, Garzón-Velásquez KB, Gil-Rondero C, Guerra-Alarcón LV, Hernández-Colín AK, Hernández-Ruiz E, Hernández-Alcántara AE, Hernández-Cervantes SA, Herrera-Olivares W, Ignacio-Ibarra G, Inclán-Alarcón SI, Leyto-Cruz F, Macías-Flores JP, Vega AM, Martínez-Ramírez MA, Martínez-Coronel J, Medina-Coral JE, Meza-Dávalos L, Montoya-Jiménez L, Morales-Hernández A, Morales-López E, Morales-Adrián JJ, Azcué MM, Mújica-Martínez A, Murillo-Cruz JL, Nájera-Martínez J, Narváez-Sarmiento IM, Nava-Villegas L, Nava-Alpide MA, Orellana Garibay JJ, Palafox-Zaldívar MT, Palma-Moreno OG, Paredes-Lozano EP, Pedraza-Colín ML, Pérez-Zúñiga JM, Pérez-Lizardi AB, Rojas-Castillejos F, Romero-Martínez E, Romero-Rodelo H, Ruiz-Contreras J, Saavedra-González A, Saucedo-Montes E, Silva-Michel LG, Silva-Vera K, Teomitzi-Sánchez Ó, Tepepa-Flores F, Ventura-Enríquez Y, Villela-Peña A, Vilchis-González SP, Zapata-Canto N, Zárate-Rodríguez PA, Alvarado-Ibarra M
Gac Med Mex 2020;156(Suppl 1):S1-S45. doi: 10.24875/GMM.M20000393. PMID: 33103663
Solitary Plasmacytoma: a Review of Diagnosis and Management.
Pham A, Mahindra A
Curr Hematol Malig Rep 2019 Apr;14(2):63-69. doi: 10.1007/s11899-019-00499-8. PMID: 30788667
Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel.
Caers J, Paiva B, Zamagni E, Leleu X, Bladé J, Kristinsson SY, Touzeau C, Abildgaard N, Terpos E, Heusschen R, Ocio E, Delforge M, Sezer O, Beksac M, Ludwig H, Merlini G, Moreau P, Zweegman S, Engelhardt M, Rosiñol L
J Hematol Oncol 2018 Jan 16;11(1):10. doi: 10.1186/s13045-017-0549-1. PMID: 29338789Free PMC Article

Diagnosis

Extramedullary Plasmacytoma: Long-Term Clinical Outcomes in a Single-Center in China and Literature Review.
Zhu X, Wang L, Zhu Y, Diao W, Li W, Gao Z, Chen X
Ear Nose Throat J 2021 May;100(4):227-232. Epub 2020 Sep 17 doi: 10.1177/0145561320950587. PMID: 32941076
Solitary Plasmacytoma: a Review of Diagnosis and Management.
Pham A, Mahindra A
Curr Hematol Malig Rep 2019 Apr;14(2):63-69. doi: 10.1007/s11899-019-00499-8. PMID: 30788667
Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel.
Caers J, Paiva B, Zamagni E, Leleu X, Bladé J, Kristinsson SY, Touzeau C, Abildgaard N, Terpos E, Heusschen R, Ocio E, Delforge M, Sezer O, Beksac M, Ludwig H, Merlini G, Moreau P, Zweegman S, Engelhardt M, Rosiñol L
J Hematol Oncol 2018 Jan 16;11(1):10. doi: 10.1186/s13045-017-0549-1. PMID: 29338789Free PMC Article
Extramedullary plasmacytoma of the small intestine: clinical features, diagnosis and treatment.
Lopes da Silva R
J Dig Dis 2012 Jan;13(1):10-8. doi: 10.1111/j.1751-2980.2011.00544.x. PMID: 22188911
Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group.
International Myeloma Working Group
Br J Haematol 2003 Jun;121(5):749-57. PMID: 12780789

Therapy

Clinical features, therapy patterns, outcomes and prognostic factors of solitary plasmacytomas: a report of the Israeli Myeloma Study Group.
Ganzel C, Trestman S, Levi S, Gatt ME, Lavi N, Vaxman I, Rouvio O, Magen H, Lebel E, Horowitz NA, Leiba M, Tadmor T, Herzog Tzarfati K, Surio C, Yeganeh S, Dally N, Avivi I, Cohen YC
Leuk Lymphoma 2022 Dec;63(14):3448-3455. Epub 2022 Sep 8 doi: 10.1080/10428194.2022.2118535. PMID: 36075048
A bispecific CAR-T cell therapy targeting BCMA and CD38 in relapsed or refractory multiple myeloma.
Mei H, Li C, Jiang H, Zhao X, Huang Z, Jin D, Guo T, Kou H, Liu L, Tang L, Yin P, Wang Z, Ai L, Ke S, Xia Y, Deng J, Chen L, Cai L, Sun C, Xia L, Hua G, Hu Y
J Hematol Oncol 2021 Oct 9;14(1):161. doi: 10.1186/s13045-021-01170-7. PMID: 34627333Free PMC Article
Primary pulmonary plasmacytoma: a case report introduction.
Nie S, Peng DC, Gong HH, Ye CL, Nie X, Li HJ
World J Surg Oncol 2016 Aug 4;14(1):205. doi: 10.1186/s12957-016-0948-8. PMID: 27487779Free PMC Article
Extramedullary plasmacytoma of the small intestine: clinical features, diagnosis and treatment.
Lopes da Silva R
J Dig Dis 2012 Jan;13(1):10-8. doi: 10.1111/j.1751-2980.2011.00544.x. PMID: 22188911
Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group.
International Myeloma Working Group
Br J Haematol 2003 Jun;121(5):749-57. PMID: 12780789

Prognosis

Extramedullary plasmacytoma: Tumor occurrence and therapeutic concepts-A follow-up.
Holler A, Cicha I, Eckstein M, Haderlein M, Pöttler M, Rappl A, Iro H, Alexiou C
Cancer Med 2022 Dec;11(24):4743-4755. Epub 2022 May 16 doi: 10.1002/cam4.4816. PMID: 35578404Free PMC Article
Analysis of the Treatment and Survival of Sinonasal Extramedullary Plasmacytoma.
Gao J, Tseng CC, Barinsky GL, Fang CH, Grube JG, Hsueh WD, Baredes S, Eloy JA
Am J Rhinol Allergy 2022 Sep;36(5):591-598. Epub 2022 Apr 19 doi: 10.1177/19458924221092529. PMID: 35440217
Solitary Plasmacytoma: a Review of Diagnosis and Management.
Pham A, Mahindra A
Curr Hematol Malig Rep 2019 Apr;14(2):63-69. doi: 10.1007/s11899-019-00499-8. PMID: 30788667
Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel.
Caers J, Paiva B, Zamagni E, Leleu X, Bladé J, Kristinsson SY, Touzeau C, Abildgaard N, Terpos E, Heusschen R, Ocio E, Delforge M, Sezer O, Beksac M, Ludwig H, Merlini G, Moreau P, Zweegman S, Engelhardt M, Rosiñol L
J Hematol Oncol 2018 Jan 16;11(1):10. doi: 10.1186/s13045-017-0549-1. PMID: 29338789Free PMC Article
Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group.
International Myeloma Working Group
Br J Haematol 2003 Jun;121(5):749-57. PMID: 12780789

Clinical prediction guides

FAM46C-mediated tumor heterogeneity predicts extramedullary metastasis and poorer survival in multiple myeloma.
Zhang W, Wu C, Geng S, Wang J, Yan C, Zhang X, Zhang JJ, Wu F, Pang Y, Zhong Y, Wang J, Fu W, Huang X, Wang W, Lyu X, Huang Y, Jing H
Aging (Albany NY) 2023 May 6;15(9):3644-3677. doi: 10.18632/aging.204697. PMID: 37155154Free PMC Article
A bispecific CAR-T cell therapy targeting BCMA and CD38 in relapsed or refractory multiple myeloma.
Mei H, Li C, Jiang H, Zhao X, Huang Z, Jin D, Guo T, Kou H, Liu L, Tang L, Yin P, Wang Z, Ai L, Ke S, Xia Y, Deng J, Chen L, Cai L, Sun C, Xia L, Hua G, Hu Y
J Hematol Oncol 2021 Oct 9;14(1):161. doi: 10.1186/s13045-021-01170-7. PMID: 34627333Free PMC Article
Clinical Features of Head and Neck Solitary Extramedullary Plasmacytoma in Taiwan.
Lai CY, Hsieh HH, Chen HK, Chao CY, Hua CH, Tai CJ, Bau DT, Tsai MH, Shih LC
In Vivo 2020 Jan-Feb;34(1):261-265. doi: 10.21873/invivo.11769. PMID: 31882487Free PMC Article
Cutaneous extramedullary plasmacytoma: clinical, prognostic, and interphase cytogenetic analysis.
Comfere NI, Gonzalez Santiago TM, Peters MS, Knudson RA, Ketterling RP, Gibson LE
Am J Dermatopathol 2013 May;35(3):357-63. doi: 10.1097/DAD.0b013e31826d729f. PMID: 23000906
Clinical outcome of extramedullary plasmacytoma.
Galieni P, Cavo M, Pulsoni A, Avvisati G, Bigazzi C, Neri S, Caliceti U, Benni M, Ronconi S, Lauria F
Haematologica 2000 Jan;85(1):47-51. PMID: 10629591

Recent systematic reviews

Diagnosis and management of extramedullary plasmacytoma in nasal cavity: Clinical experience and literature review.
Hu H, Hu X, Hu G, Li D, Cai J
Medicine (Baltimore) 2023 Jan 13;102(2):e32647. doi: 10.1097/MD.0000000000032647. PMID: 36637932Free PMC Article
Plasma Cell Myeloma - 20-Year Comparative Survival and Mortality of Three Plasma Cell Myeloma ICD-O-3 Oncologic Phenotypes by Age, Sex, Race, Stage, Cohort Entry Time-Period and Disease Duration: A Systematic Review of 111,041 Cases for Diagnosis Years 1973-2014: (SEER*Stat 8.3.4).
Milano AF
J Insur Med 2018;47(4):203-211. Epub 2019 Jan 22 doi: 10.17849/insm-47-04-1-9.1. PMID: 30668210
Pattern of care and impact of prognostic factors on the outcome of head and neck extramedullary plasmacytoma: a systematic review and individual patient data analysis of 315 cases.
Venkatesulu B, Mallick S, Giridhar P, Upadhyay AD, Rath GK
Eur Arch Otorhinolaryngol 2018 Feb;275(2):595-606. Epub 2017 Dec 9 doi: 10.1007/s00405-017-4817-z. PMID: 29224044
Sinonasal extramedullary plasmacytoma: a systematic review of 175 patients.
D'Aguillo C, Soni RS, Gordhan C, Liu JK, Baredes S, Eloy JA
Int Forum Allergy Rhinol 2014 Feb;4(2):156-63. Epub 2013 Dec 13 doi: 10.1002/alr.21254. PMID: 24339430
Management of multiple myeloma and related-disorders: guidelines from the Italian Society of Hematology (SIE), Italian Society of Experimental Hematology (SIES) and Italian Group for Bone Marrow Transplantation (GITMO).
Barosi G, Boccadoro M, Cavo M, Corradini P, Marchetti M, Massaia M, Merlini G, Tosi P, Tura S; Italian Society of Hematology; Italian Society of Experimental Hematology; Italian Group for Bone Marrow Transplantation
Haematologica 2004 Jun;89(6):717-41. PMID: 15194540

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